(1) Fibroblasts from two such TSD patients had Hex A activity comprising 16% of total Hex when measured by thermal fractionation and quantitation with 4-methylumbelliferyl-beta-D-N-acetylglucosamine (4MUG).
(2) We have shown previously that a mutant sequence (hex-3), which differs from hex-1 by three base pairs, can no longer bind these two factors significantly.
(3) About 200 urine samples, from the various categories listed above, were analyzed for Hex A with both the heat denaturation and pH inactivation methods and the results compared with serum and leukocyte levels from many of the same individuals.
(4) Hexosaminidase in extracts from normal kidney and renal carcinoma tissue could be separated into two major forms [hexosaminidase A (Hex A) and hexosaminidase B (Hex B)] by Cellogel electrophoresis or by diethylaminoethyl cellulose column chromatography.
(5) This finding also suggests that the Hex, Mut, and PMS systems evolved from a common ancestor and that functionally similar mismatch repair systems could be widespread among procaryotic as well as eucaryotic organisms.
(6) The greatest amount of hex S activity was found in fibroblast, but it was also observed in lesser amounts in liver, kidney, intestine and placenta.
(7) At low pH, two of the latter mabs reacted only with Hex B, and it was, therefore, possible to develop enzyme immunoassays for the specific determination of Hex A and Hex B.
(8) The competitive ganglionic nicotinic blocking agent, HEX, on the other hand, inhibited both the increased locomotion produced by CRF in the home cage and also the decreased locomotion induced by CRF in the modified open field.
(9) Hex 70-Inv and Hex 532-Inv, like endogenous lysosomal enzymes, were subject to developmentally induced secretion.
(10) A hexose (Hex)2-3 branch is attached to the heptose linked directly to KDO and a GalNAc-Hex3, GlcNAc, and PEA are separately attached to the second heptose.
(11) The commonly used screening test for detection of Tay-Sachs disease (TSD) carriers is the serum Hex heat inactivation method (HIM), which is based on the assumption that Hex A is the only thermolabile component of Hex; However, in the presence of thermolabile Hex B, HIM could lead to false-negative results.
(12) HEX(C) is shown to differ from HEX(A) and HEX(B) in substrate specificity, molecular size and affinity for Concanavalin-A.
(13) The activities of beta-hexosaminidase (HEX), a lysosomal glycosidase, were studied in serum (sHEX) and urine (uHEX) among men with various drinking habits.
(14) The Hex treated dogs and cats exhibited a fall of MAP and CBF with enhancement of CVR.
(15) The electrophoretic pattern of human fibroblast extracts displays three bands (cathode to anode) of hexosaminidase: Hex B, Hex A, Hex C in normal strains, only one B band in Tay-Sachs strains, and only one C band in Sandhoff strains.
(16) Though monkey tissues also cross-reacted with human seminal plasma Hex-antisera, mouse, rat, and goat testis and epididymis extracts failed to show any cross-reaction.
(17) Hex S, the major residual beta hexosaminidase activity present in tissues, fluids, and cultured skin fibroblasts of patients with type 0 GM2 gangliosidosis, was isolated and characterized biochemically and immunologically.
(18) The persistence of a second major hexosaminidase isozyme, HEX B, does not protect against the lethal accumulation of GM2 ganglioside in the central nervous system.
(19) The results suggest that, in the patient's fibroblasts, a defect in beta-chain synthesis or processing precludes the self-association of beta-chains to form Hex B, but does not prevent the association of alpha- and beta-chains to form Hex A.
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Hox
Definition:
(v. t.) To hock; to hamstring. See Hock.
Example Sentences:
(1) In a previous analysis of the Hox-1.1 promoter in transgenic mice, we identified sequences that were sufficient to establish transgene expression in a specific region of the embryo.
(2) Thus, Hox-1.6 is involved in regional specification along the rostrocaudal axis, but only in its most rostral domain of expression.
(3) The mouse Hox 2.1 gene contains a homeobox sequence and is therefore a candidate for a vertebrate gene involved in the control of embryonic patterning or positional specification.
(4) The results show that the Hox 2.1 gene produces at least two related phosphorylated proteins present in different proportions in different tissues.
(5) Two of the hox appear to be cognates of the human Hu-1 (or mouse Hox 2.1) and the mouse Hox 1-3, while another is closely related to the mouse Hox 1-4.
(6) The expression of the eleven HOX 1 genes is lineage-restricted and these genes are predominantly detected within cells of myelomonocytic origin.
(7) Our analysis of Hox 1.3 cDNA and genomic clones indicates that the gene is organized into two exons and encodes a 270-amino-acid homeo domain protein.
(8) Southern blotting shows that the DNA sequence encoding Hox-6.1 is single copy.
(9) A 2.4 kb RNA encoded by the murine Hox 1.1 (m6) homeobox gene is induced when F9 stem cells are differentiated with retinoic acid and dibutyryl cyclic AMP.
(10) This report demonstrates that the genes in the murine Hox-2 cluster display spatially and temporally dynamic patterns of expression in the transverse plane of the developing CNS.
(11) The early transcription pattern thus appears to be compatible with a regionalizing role for the Hox-3.1 gene.
(12) Here we show correct regulation by Hox-1.1 sequences in transgenic mice and identify the elements responsible for different levels of control.
(13) The resulting Hox-2.2 misexpression produces early postnatal lethality as well as craniofacial and axial skeletal perturbations that include open eyes at birth, cleft palate, micrognathia, microtia, skull bone deficiencies, and structural and positional alterations in the vertebral column.
(14) This article reviews recent studies on the expression of the homeobox gene, Hox 2.1, during mouse embryogenesis, using the technique of in situ hybridization.
(15) In situ hybridization shows that Hox 2.1 transcripts are regionally localized in the spinal cord in an apparent anterior-posterior gradient extending from the hind brain.
(16) In this review we have built a case for abnormal Hox gene expression in human congenital malformations without presenting any direct evidence of their involvement.
(17) Hox 2.3 was also super-induced by serum, in the presence of cycloheximide, in cells rested previously in serum-free media, suggesting that new protein synthesis was not required for expressive augmentation.
(18) To further understand their regulation, the genomic loci of four newt HBox genes (two from the human HBox (HOX)-2 complex and two from the HOX-3 complex) were analysed and compared with homologous loci in other vertebrates.
(19) DNase I protection of the Hox-1.3 gene promoter region with the Hox-1.3 protein identifies a binding site 144 bp upstream from the start of transcription.
(20) The homeodomain is identical to the genomic sequence for Hox 1H, a newly identified member of the Hox 1 locus (Acampora et al.